Medical Definition of Chediak-Steinbrinck-Higashi syndrome

1. An autosomal recessive disorder characterised by the presence of giant lysosomal vesicles in phagocytes and in consequence poor bactericidal function due to deficient secretion of myeloperoxidase by lysosomes. There is some perturbation of microtubule dynamics. There are abnormalities of granulation and nuclear structure of all types of leukocytes with malformation of peroxidase-positive granules, cytoplasmic inclusions, and Dohle bodies, often with hepatosplenomegaly, lymphadenopathy, anaemia, thrombocytopenia, roentgenologic changes of bones, lungs and heart, skin and psychomotor abnormalities, and susceptibility to infection. The condition usually results in death in childhood, before the age of 10. Reported from humans, albino Hereford cattle, mink, beige mice and killer whale. Compare: chronic granulomatous disease. Inheritance: autosomal recessive. Synonym: Beguez Cesar disease, Chediak-Higashi disease, Chediak-Steinbrinck-Higashi anomaly. (21 May 1997)

Lexicographical Neighbors of Chediak-Steinbrinck-Higashi Syndrome

Chechenia
Chechens
Chechneya
Chechnia
Chechniya
Chechnya
Cheddar
Cheddar cheese
Cheddar cheeses
Cheddarhead
Cheddarheads
Cheddaring
Chediak-Higashi disease
Chediak-Higashi syndrome
Chediak-Steinbrinck-Higashi anomaly
Chediak-Steinbrinck-Higashi syndrome (current term)
Cheek
Cheerio
Cheerio effect
Cheerios
Cheetos
Cheever
Chehalem berries
Chehalem berry
Chehalem blackberry
Cheilanthes
Cheilanthes alabamensis
Cheilanthes eatonii
Cheilanthes gracillima
Cheilanthes lanosa

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